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Ann Thorac Surg 1995;59:301-304
© 1995 The Society of Thoracic Surgeons

Successful Application of the Norwood Procedure for Infants Without Hypoplastic Left Heart Syndrome

Emory University School of Medicine and Egleston Children's Hospital at Emory University, Atlanta, Georgia

	Abstract

Top Footnotes Abstract Introduction Patients and Methods Results Comment Addendum References

 
Although the first-stage Norwood procedure mostly has been used for hypoplastic left heart syndrome, there are other anomalies in which the Norwood procedure can be applied. Since 1991, 18 newborns without hypoplastic left heart syndrome underwent a first-stage Norwood procedure. All had a hypoplastic aortic annulus, ascending aorta, and transverse aorta. Ten had normally related great arteries: aortic atresia or aortic stenosis with inadequate left ventricle in 4, mitral atresia or stenosis in 4, and interrupted aortic arch in 2. The 8 others had double-outlet right ventricle with mitral atresia or complete transposition with a hypoplastic right ventricle. Age ranged from 2 to 77 days (median, 6 days) and weight from 2.4 to 4.4 kg (mean, 3.0 kg). The patients with interrupted aortic arch simultaneously underwent primary repair of the interruption. There were 17 hospital survivors (94%). There have been no late deaths in follow-up from 4 to 30 months (mean, 15.5 months). Thirteen children have had subsequent creation of a bidirectional Glenn shunt with takedown of the original systemic to pulmonary shunt. The 2 with interrupted aortic arch underwent a Rastelli-type biventricular repair. These results show that the Norwood procedure can be applied to infants without hypoplastic left heart syndrome who have hypoplastic aortas and excessive pulmonary blood flow with very low mortality and excellent palliation.

	Introduction

Top Footnotes Abstract Introduction Patients and Methods Results Comment Addendum References

 
See also page 304.

Over the past decade, impressive advances in the surgical palliation of infants with hypoplastic left heart syndrome have been achieved largely due to the pioneering work of Norwood and his colleagues [1, 2]. Physiologically, there are anatomic lesions other than classic hypoplastic left heart syndrome characterized by excessive pulmonary blood flow and hypoplasia of the aorta in which the principles of the Norwood procedure can be applied [3–5]. Because most of these infants functionally have a single ventricle, which eventually will require a Fontan procedure as definitive palliation, early surgical intervention should be aimed at protecting the pulmonary vasculature and establishing unobstructed systemic blood flow while avoiding increased afterload, which could lead to myocardial fibrosis and ventricular noncompliance. With this in mind, we have applied the first-stage Norwood procedure to 18 infants without classic hypoplastic left heart syndrome at Egleston Children's Hospital at Emory University; these children form the basis of this report.

	Patients and Methods

Top Footnotes Abstract Introduction Patients and Methods Results Comment Addendum References

 
Patient Population
From January 1991 to July 1993, 18 consecutive infants (8 boys, 10 girls) without classic hypoplastic left heart syndrome but with a hypoplastic aortic annulus (defined as 5 mm) [6], ascending aorta, and transverse aorta underwent a first-stage Norwood procedure (Table 1). Ages ranged from 2 to 77 days (median, 6 days) with all but 3 patients less than 2 weeks old. Weight ranged from 2.4 to 4.4 kg (mean, 3.0 kg). Ten patients had normally related great arteries, 2 with type B interrupted aortic arch and subaortic obstruction, 4 with mitral stenosis or atresia, and 4 with aortic stenosis or atresia with an inadequate left ventricle (left ventricular end-diastolic volume <20 cm³/m²) [7]. Two patients had double-outlet right ventricle with mitral atresia, ventricular septal defect, and hypoplastic left ventricle. The remaining 6 infants had transposition of the great arteries with a hypoplastic right ventricle, 4 with tricuspid stenosis or atresia, and 2 with double-inlet left ventricle. In addition, 3 patients had anomalies of systemic venous return with interrupted inferior vena cava with azygos continuation to the superior vena cava in patient 2 and bilateral superior venae cavae without an innominate vein in patients 12 and 16. Patient 1 with type B interrupted aortic arch had DiGeorge's syndrome.

Pulmonary blood flow was established using either a 3.5-mm (2 patients) or 4.0-mm (16 patients) polytetrafluoroethylene shunt as a right modified Blalock-Taussig shunt (12 patients) or as a central shunt from the neo-aorta to the right pulmonary artery (6 patients). The defect in the distal pulmonary trunk was closed with a homograft patch in each patient.

Postoperative Care
To prevent pulmonary overcirculation and systemic hypoperfusion in the early postoperative period, efforts were made to avoid reducing pulmonary vascular resistance. This was achieved by low inspired oxygen concentration as long as the arterial oxygen saturation was maintained at 70% or greater. All patients were weaned to room air before leaving the operating room. Carbon dioxide was added to the inspired gas mixture up to a concentration of 3% to achieve an arterial carbon dioxide tension of 45 to 55 mm Hg [9]. The hematocrit was maintained between 45% to 50% and ionized calcium levels kept at 4.5 to 5.0 mg/dL using an infusion of calcium gluconate. Dopamine was used most commonly for inotropic support.

	Results

Top Footnotes Abstract Introduction Patients and Methods Results Comment Addendum References

 
Hospital Survival
There were 17 hospital survivors (94%) with one death in patient 17 related to myocardial ischemia from a technical error during construction of the neo-aorta with kinking of the diminutive ascending aorta. Although the problem was recognized in the operating room and corrected during a second period of circulatory arrest, myocardial function never adequately recovered despite high-dose inotropic support; the child died on the first postoperative day of progressive low cardiac output.

Patient 3 required delayed sternal closure on the fourth postoperative day due to excessive mediastinal swelling at the original operation. Need for inotropic support ranged from 2 to 8 days (mean, 2.9 days) and mechanical ventilation from 2 to 6 days (mean, 4 days). Patient 1 required right hemidiaphragm plication on the fifth postoperative day due to right phrenic nerve injury. Patient 4 had addition of a 4-mm left modified Blalock-Taussig shunt on the twenty-eighth postoperative day because of inadequate pulmonary blood flow from his original 4-mm central shunt. There was one episode of mediastinitis requiring pectoralis muscle flap closure in patient 18. He subsequently has undergone a bidirectional Glenn shunt with takedown of his original right modified Blalock-Taussig shunt with primary sternal closure at 7 months of age. Initial hospitalization in this series ranged from 7 to 50 days (mean, 18 days).

Follow-up
The 17 operative survivors have been followed up for 4 to 30 months (mean, 15.5 months) with no late deaths. Both patients with interrupted aortic arch have undergone successful biventricular repair using a Rastelli-type repair consisting of resection of the infundibular septum, closure of the ventricular septal defect baffling left ventricular blood through the aortic and pulmonary valves to the neo-aorta, and construction of right ventricular to pulmonary artery continuity with a homograft valved conduit.

Two patients have required balloon dilation of recurrent coarctation, 1 clearly related to failure during the original operation to take the homograft patch distal to the insertion of the ductus arteriosus on the descending aorta.

Of the 15 operative survivors with a functional single ventricle, 13 have undergone successful takedown of the systemic to pulmonary shunt with creation of a bidirectional Glenn shunt from 4 to 11 months (mean, 8.5 months) postoperatively in anticipation of an eventual Fontan procedure. The remaining 2 patients are awaiting a Glenn shunt now at 5 and 6 months of age. Patient 15 required catheter ablation of an accessory conduction pathway early after his bidirectional Glenn shunt because of refractory malignant supraventricular tachycardia. To date, only 1 patient (patient 4) has been determined to be an unsuitable candidate for a Fontan procedure due to poor ventricular function and tricuspid regurgitation. He is currently being evaluated for cardiac transplantation.

	Comment

Top Footnotes Abstract Introduction Patients and Methods Results Comment Addendum References

 
Initial surgical management of infants with hypoplastic aortas and unrestricted pulmonary blood flow is quite problematic. The spectrum of lesions in such children without classic hypoplastic left heart syndrome can be associated with a variety of other anomalies including abnormalities of great arterial relationships, atresia or stenosis of either atrioventricular valve, or hypoplasia of either of the ventricles. Early palliation should be directed at relieving aortic obstruction, protecting the pulmonary circulation, and preventing the deleterious effects of chronic pressure or volume overload on the ventricle.

The infants presented in this series represent a heterogeneous group of patients managed with a common initial surgical procedure. Patients 1 and 2 in this series with interrupted aortic arch and left ventricular outflow tract obstruction were the only 2 with suitable anatomy (two usable atrioventricular valves, two normal-sized ventricles) for eventual biventricular repair. Although Bove and associates [10] have recommended resection of the subaortic obstruction at the time of primary repair, others have reported a high mortality in patients with interrupted aortic arch and left ventricular outflow tract obstruction undergoing attempted primary repair [7, 11]. The staged approach used in our 2 patients (Norwood procedure followed by Rastelli procedure) did not require a prosthetic conduit to reconstruct aortic continuity as advocated by others [12, 13] but of course has the disadvantage of the use of a homograft valved right ventricular to pulmonary artery conduit, which eventually will need to be replaced as the child outgrows it.

The remaining 16 patients in this series had a functionally single ventricle and thus are candidates for an eventual Fontan procedure. There have been many reports describing the detrimental long-term effects of pulmonary artery banding in such patients related to aggravation of subaortic obstruction and reduction in myocardial compliance, which is so crucial for the success of an eventual Fontan procedure [14–16]. Recently, some authors even have advocated avoiding the use of pulmonary artery banding by employing the arterial switch procedure with reconstruction of the hypoplastic aorta in these patients [17, 18].

Borrowing from the increasingly favorable results with the Norwood procedure for palliation of classic hypoplastic left heart syndrome [8], some have applied the Norwood procedure to these patients [3–5] as has been presented in this series. The Philadelphia group in 1991 reported a 68% 30-day survival in 34 infants with severe aortic outflow tract obstruction and lesions other than hypoplastic left heart syndrome treated with a Norwood procedure [5]. The Boston group described a 63% early survival rate in 16 infants with critical aortic stenosis or mitral stenosis palliated with a Norwood procedure [19]. Although the present report compares quite favorably with these series (94% hospital survival), the rapid evolution and constant improvements in operative and postoperative management of these patients probably accounts for the better results in our more recent series.

It is very encouraging that late complications in this series have been few (only 2 patients with recoarctation) and that 13 of the 15 survivors (87%) with functionally single ventricles already have undergone successful conversion to a bidirectional Glenn shunt in preparation for an eventual Fontan procedure. In only 1 of these patients to date has ventricular dysfunction and tricuspid regurgitation severe enough to preclude serious consideration for a Fontan procedure developed. The important question yet to be answered, however, is how these patients will fare with completion of their Fontan procedure. Based on previous experience in children with hypoplastic left heart syndrome [2], there is reason to be optimistic.

In summary, we have shown that a heterogeneous group of neonates with aortic obstruction and unrestricted pulmonary blood flow without classic hypoplastic left heart syndrome can undergo successful palliation with the Norwood procedure with low morbidity and mortality. This procedure allows for adequate relief of aortic obstruction, protection of the pulmonary vasculature, and preservation of myocardial function, which hopefully will facilitate later corrective operations.

	Addendum

Top Footnotes Abstract Introduction Patients and Methods Results Comment Addendum References

 
Since original submission of this article, all of the 15 operative survivors without interrupted aortic arch have successfully undergone a bidirectional Glenn shunt, of whom 3 have undergone a successful modified Fontan procedure. The 1 child listed for cardiac transplantation underwent successful orthotopic cardiac transplantation. There continue to be no late deaths on follow-up of 16 to 42 months (mean, 27 months).

	Footnotes

Top Footnotes Abstract Introduction Patients and Methods Results Comment Addendum References

 
Presented at the Fortieth Annual Meeting of the Southern Thoracic Surgical Association, Panama City Beach, FL, Nov 4–6, 1993.

Address reprint requests to Dr Kanter, Division of Cardiothoracic Surgery, Emory University, 1365 Clifton Rd, NE, Atlanta, GA 30322.

	References

Top Footnotes Abstract Introduction Patients and Methods Results Comment Addendum References

 

1. Norwood WI, Lang P, Hansen DD. Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med 1983;308:23–6.[Medline]
2. Norwood WI, Jacobs ML, Murphy JD. Fontan procedure for hypoplastic left heart syndrome. Ann Thorac Surg 1992;54:1025–30.[Abstract]
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4. Imai Y, Kurosawa H, Fujiwara T, et al. Palliative repair of aortic atresia associated with tricuspid atresia and transposition of the great arteries. Ann Thorac Surg 1991;51:646–8.[Abstract]
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